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Madisons Foundation - Moms And Dads In Search Of Needed Support
What
Astrocytoma is a general term applied to a wide range of neoplasms, or abnormal tissue growth called "tumors," affecting the central nervous system (CNS). Astrocytoma is the most common type of brain tumor in children. The tumor is composed of astrocytes, which are special star-shaped cells unique to the CNS. These tumors can vary in their location, clinical course, extent of invasiveness, growth potential, tendency for progression, and morphology or structural form, and are characterized as "low-grade" or "high-grade" accordingly. Most astrocytomas are indolent, low-grade tumors that arise along midline structures of the CNS, such as the cerebellum, and a region called the diencephalons, which contains the hypothalamus and visual pathway. Astrocytomas of the midbrain and medulla are also most likely to be low-grade. The remaining high-grade tumors are malignant, meaning that they worsen over time and resist treatment. These most typically arise in the cerebral hemispheres or certain parts of the brainstem. Spinal cord astrocytomas are the least common, but these can be either low-grade or high-grade.Who
Astrocytomas account for the majority of pediatric brain tumors. The peak age of children with astrocytoma is between the ages of 5 and 10. There is no difference in incidence between boys and girls and the disease appears to affect all races equally. Studies suggest that the annual incidence in the United States is approximately 14 new cases per million children under the age of 15. Supratentorial and cerebellar astrocytomas comprise about 50% of childhood brain tumors. In general younger children tend to develop more infratentorial tumors (cerebellar).
Signs and Symptoms
Children often exhibit symptoms for many months prior to diagnosis. Major symptoms may include:
- Headaches (characterized by pain early in the morning that lessens during the day and is relieved by vomiting).
- Early morning vomiting
- New onset seizures
- Visual complaints or other localized neurologic abnormalities due to the increased intracranial pressure and local tumor effect.
- Children with cerebellar astrocytomas may also exhibit nystagmus (repetitive eye movements) or ataxia (unsteadiness of gait). These children will hold their feet apart in order to steady themselves when they attempt to walk.
- Older children often complain of fatigue or motor weakness.
In some cases, affected individuals exhibit personality changes.
Possible Causes
At present, there is no single cause of astrocytoma. However, some research suggests that this tumor is associated with a mutation, or permanent change, in a particular gene called neurofibromatosis 1 (NF1).
Diagnosis
The child's history is a major part of making the diagnosis as are the findings from the physical examination A thorough neurological examination may help pinpoint the location of the mass. Examination of the cerebrospinal fluid (CSF) may also be performed if there is no increased intracranial pressure. Collectively, these give strong indication of the underlying problem, but the diagnosis can be substantiated with various imaging studies, including computed tomography (CT) scans and magnetic resonance imaging (MRI) scans. MRI scans allow for better visualization of the back of the brain. Once the tumor is detected, a biopsy of the mass is performed and special tissue stains are used to determine the specific grade or subtype. If seizures are present, an electroencephalogram (EEG) can be performed to evaluate and monitor seizure activity.
Treatment
Chemotherapy, radiotherapy and surgical(complete or partial) resection may be used depending on the location and grade of tumor. Surgical resection is the primary treatment for low-grade tumors. A complete resection is preferable as this minimizes the chance for recurrence. However, some low-grade tumors are also inoperable or may be only partially resected. In these cases, treatment may include radiotherapy. For high-grade tumors, surgical resection is also attempted, followed by irradiation. Chemotherapy has had limited success in treating astrocytoma because of the minimal impact on the overall survival of patients with high-grade tumors. In cases of low-grade tumors, it is often administered to those with inoperable tumors, such as those located in the diencephalon or brainstem. Tumors in this location are considered inoperable because of the low chances of survival associated with their removal. For some children with increased intracranial pressure, placement of a shunt that drains the CSF may be necessary. This shunt is often temporary but can be prone to malfunction. The age of the child is also a factor affecting treatment choices, and in some cases, chemotherapy may be preferred to radiotherapy. The variety of treatments involved in astrocytoma means that these children will have a variety of doctors working together to help deliver the best care. These specialists include pediatric oncologists, neurologists, and neurosurgeons, among others. Physical therapists also work with affected children to prevent muscle wasting and significant disability.
Prognosis
The prognosis for astrocytoma varies considerably based on the grade and location of the tumor. Low-grade tumors are associated with better prognoses than high-grade tumors. The best prognosis is observed in children with low-grade astrocytomas that were treated with complete resection: the ten-year survival rate for these children is nearly 100%. For all low-grade astrocytomas, the equivalent survival rates are 60-95%. Depending on the exact location, children may exhibit residual motor deficits and seizure disorder. Those who survive high-grade astrocytoma are often left with some degree of dysfunction.
Connect with other parents
In the spirit of community and support, Madisons Foundation offers the unique service of connecting parents of children with rare diseases. If you would like to be connected to other parents of children with this disease, please fill out this brief form.Weblinks
American Brain tumor Society
This is an excellent site for both patients and health professionals. Offers comprehensive information pertaining to tumors, treatment and clinical trials, as well as additional resources and support services. Has a special link for kids.
The Brain tumor Society
Another thorough site devoted to education, research and support.
National Brain tumor Foundation
This organization is focused on offering support services. The site invites patients and their families to participate in the nationwide Angel Adventures.
National Institute of Neurologic Disorders and Stroke tumor_hope_through_research.htm
A very thorough and informative site sponsored by the National Institute of Neurological Disorders and Stroke that is easy to navigate.